Biochemistry · Biomolecules and Proteins

Phenylalanine and tyrosine metabolism with inborn errors

5 min read

Quick Orientation

Section

Definition

Objective
Understand the core idea
Keywords
Phenylalanine, tyrosine, glucogenic and ketogenic

Conversion of Phenylalanine to Tyrosine

Objective
Learn the main facts and relationships
Keywords
Phenylalanine hydroxylase, tetrahydrobiopterin (H4-biopterin), NADPH-dependent dihydrobiopterin reductase

Degradative Pathway and Its Inborn Errors

Objective
Follow the sequence and key control points
Keywords
homogentisate, fumarate, acetoacetate

Melanin, Thyroid Hormones and Catecholamines

Objective
Learn the main facts and relationships
Keywords
melanin, thyroid hormones, catecholamines

Catecholamine Synthesis and Functions

Objective
Follow the sequence and key control points
Keywords
Tyrosine hydroxylase, dopamine, norepinephrine

Clinical Correlation

Objective
Connect the mechanism to clinical findings
Keywords
Phenylketonuria (PKU), 20 to 65 mg/dL, 1 to 2 mg/dL

Definition